复旦大学附属中山医院：《内科学》课程教学资源（PPT讲稿）陈勤奋－溶血性贫血 Hemolytic Anemia

溶血性贫血 Hemolytic Anemia 华山医院血液科 陈勤奋

溶血性贫血 Hemolytic Anemia 华山医院血液科 陈勤奋

HEMOLYTIC ANEMIA (reduced RBC life span) Anemia of increased destruction CRNormochrom1c. normochromic anemia a& Shortened rBC survival cRReticulocytosis--Response to increased rbc destruction cRIncreased indirect bilirubin iNCreased LDh

HEMOLYTIC ANEMIA (reduced RBC life span) ❖Anemia of increased destruction Normochromic, normochromic anemia Shortened RBC survival Reticulocytosis--Response to increased RBC destruction Increased indirect bilirubin Increased LDH

Mechanisms and Causes 令Ⅰ NTRACORPUSCULAR HEMOLYSIS mEMbrane Abnormalities METabolic abnormalities heMoglobinopathies EXTRACORPUSCULAR HEMOLYSIS nONimmune ORImune

Mechanisms and Causes ❖INTRACORPUSCULAR HEMOLYSIS Membrane Abnormalities Metabolic Abnormalities Hemoglobinopathies ❖EXTRACORPUSCULAR HEMOLYSIS Nonimmune Immune

Membrane defects Microskeletal defects cOHereditary spherocytosis o Increased sensitivity to complement paRoxysmal nocturnal hemoglobinuria

Membrane Defects ❖Microskeletal defects Hereditary spherocytosis ❖Increased sensitivity to complement Paroxysmal nocturnal hemoglobinuria

Enzymopathies % Glucose 6-Phosphate Dehydrogenase Deficiency o Pyruvate Kinase Deficiency

Enzymopathies ❖Glucose 6-Phosphate Dehydrogenase Deficiency ❖Pyruvate Kinase Deficiency

Hemoglobinopathies 冷 Hemoglobinopathy ☆ Thalassemia

Hemoglobinopathies ❖Hemoglobinopathy ❖Thalassemia

Extracorpuscular Hemolysis Nonimmune ☆ Infectious Chemical 令 Therma1 ☆0 smotic

Extracorpuscular Hemolysis Nonimmune ❖Infectious ❖Chemical ❖Thermal ❖Osmotic

Extracorpuscular Hemolysis Immune g All require antigen-antibody reactions Types of reactions dependent on RR Class of antibody cRNumber spacing of antigenic sites on cell aVAilability of complement cR Environmental Temperature c& Functional status of reticuloendothelial system ☆ Manifestations c&Intravascular hemolysis eXTravascular hemolysis

Extracorpuscular Hemolysis Immune ❖ All require antigen-antibody reactions ❖ Types of reactions dependent on: Class of Antibody Number & Spacing of antigenic sites on cell Availability of complement Environmental Temperature Functional status of reticuloendothelial system ❖ Manifestations Intravascular hemolysis Extravascular hemolysis

Extracorpuscular Hemolysis Immune Antibodies combine with rbc.& either 1. Activate complement cascade, &/or 2. Opsonize rbc for immune system .o If l, if all of complement cascade is fixed to red cell, intravascular cell lysis occurs &s If 2, &/or if complement is only partially fixed macrophages recognize Fc receptor of ig &/or C3b of complement phagocytize RBC, causing extravascular rbc destruction

Extracorpuscular Hemolysis Immune ❖ Antibodies combine with RBC, & either 1. Activate complement cascade, &/or 2. Opsonize RBC for immune system ❖ If 1, if all of complement cascade is fixed to red cell, intravascular cell lysis occurs ❖ If 2, &/or if complement is only partially fixed, macrophages recognize Fc receptor of Ig &/or C3b of complement & phagocytize RBC, causing extravascular RBC destruction

Classification Intravascular 令 Extravascular

Classification ❖Intravascular ❖Extravascular